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Conference Paper: Does in-utero transfusion for homozygous α0-thalassemia depend on hemoglobin level alone?

TitleDoes in-utero transfusion for homozygous α0-thalassemia depend on hemoglobin level alone?
Authors
Issue Date2009
PublisherJohn Wiley & Sons Ltd. The Journal's web site is located at http://www.interscience.wiley.com/jpages/0960-7692/
Citation
The 19th World Congress on Ultrasound in Obstetrics and Gynecology, Hamburg, Germany, September 13–17 2009. In Ultrasound in Obstetrics and Gynecology, 2009, v. 34 n. S1, p. 249 Abstract no. P18.08 How to Cite?
AbstractObjectives: To determine hematological parameters in fetusesaffected by α0-thalassemiaMethods: This was a retrospective cross-sectional study of 546 fetalor cord blood collected at 12–40 weeks’ gestation for the prenataldiagnosis of α0-thalassemia or other indications from 1993 to 2006.The proportion of hemoglobin (Hb) fractions was determined byelectrophoresis of hemolysate on cellulose acetate.Results: In homozygous α0-thalassemia (n = 183), the median Hblevel, proportion of Hb Bart’s(γ4) and Hb Portland 1(ξ2γ2)were6.4 g/dl, 77.5% and 22.5% respectively. While the Hb level andthe proportion of Hb Bart’s increased significantly with gestation,the proportion of Hb Portland 1 (ξ2γ2) decreased. Although mildanemia (with reference to the gestation) was present in 27.5% of the affected fetuses, the mean Hb level contributed by HbPortland 1(ξ2γ2), with normal ability to release oxygen, was only1.4 g/dl from 12 to 40 weeks’ gestation. There were no differences inthe hematological parameters between hydropic and non-hydropicfetuses. In one affected case, the middle cerebral artery peaksystolic velocity (MCAPSV) was 49cm/s (> 1.5MoM) at gestation of21 weeks. Cordocentesis showed Hb level of 6.5 g/dl, hematocrit of0.266, pO2 of 6.2 kPa, and pH of 7.42. First in-utero transfusion(IUT) was given. At 29 weeks’ gestation, MCAPSV was increasedagain to 56 cm/s (> 1.5MoM). Cordocentesis showed mild anemia(Hb of 9.1g/dl), but acidosis (pH of 7.35) and hypoxia (pO2 of3.4kPa). After correction of fetal anemia by the second IUT, boththe fetal pH (7.43) and pO2 (6.9 kPa) returned to normal. Theaffected baby was subsequently delivered at term, and survived.Conclusion: Although the degree of anemia is mild in around one-quarter of the affected fetuses, the Hb level contributed by HbPortland 1(ξ2γ2) is very low in all affected fetuses from 12 weeks’gestation. Whether to give IUT to an affected fetus depends not juston fetal Hb level, but also on ultrasound signs of severe anemia,oxygen and pH status.
Persistent Identifierhttp://hdl.handle.net/10722/224434
ISSN
2015 Impact Factor: 4.197
2015 SCImago Journal Rankings: 1.766

 

DC FieldValueLanguage
dc.contributor.authorLi, TKT-
dc.contributor.authorLeung, KY-
dc.contributor.authorLam, YH-
dc.contributor.authorTang, MHY-
dc.contributor.authorChan, VNY-
dc.date.accessioned2016-04-05T04:33:27Z-
dc.date.available2016-04-05T04:33:27Z-
dc.date.issued2009-
dc.identifier.citationThe 19th World Congress on Ultrasound in Obstetrics and Gynecology, Hamburg, Germany, September 13–17 2009. In Ultrasound in Obstetrics and Gynecology, 2009, v. 34 n. S1, p. 249 Abstract no. P18.08-
dc.identifier.issn0960-7692-
dc.identifier.urihttp://hdl.handle.net/10722/224434-
dc.description.abstractObjectives: To determine hematological parameters in fetusesaffected by α0-thalassemiaMethods: This was a retrospective cross-sectional study of 546 fetalor cord blood collected at 12–40 weeks’ gestation for the prenataldiagnosis of α0-thalassemia or other indications from 1993 to 2006.The proportion of hemoglobin (Hb) fractions was determined byelectrophoresis of hemolysate on cellulose acetate.Results: In homozygous α0-thalassemia (n = 183), the median Hblevel, proportion of Hb Bart’s(γ4) and Hb Portland 1(ξ2γ2)were6.4 g/dl, 77.5% and 22.5% respectively. While the Hb level andthe proportion of Hb Bart’s increased significantly with gestation,the proportion of Hb Portland 1 (ξ2γ2) decreased. Although mildanemia (with reference to the gestation) was present in 27.5% of the affected fetuses, the mean Hb level contributed by HbPortland 1(ξ2γ2), with normal ability to release oxygen, was only1.4 g/dl from 12 to 40 weeks’ gestation. There were no differences inthe hematological parameters between hydropic and non-hydropicfetuses. In one affected case, the middle cerebral artery peaksystolic velocity (MCAPSV) was 49cm/s (> 1.5MoM) at gestation of21 weeks. Cordocentesis showed Hb level of 6.5 g/dl, hematocrit of0.266, pO2 of 6.2 kPa, and pH of 7.42. First in-utero transfusion(IUT) was given. At 29 weeks’ gestation, MCAPSV was increasedagain to 56 cm/s (> 1.5MoM). Cordocentesis showed mild anemia(Hb of 9.1g/dl), but acidosis (pH of 7.35) and hypoxia (pO2 of3.4kPa). After correction of fetal anemia by the second IUT, boththe fetal pH (7.43) and pO2 (6.9 kPa) returned to normal. Theaffected baby was subsequently delivered at term, and survived.Conclusion: Although the degree of anemia is mild in around one-quarter of the affected fetuses, the Hb level contributed by HbPortland 1(ξ2γ2) is very low in all affected fetuses from 12 weeks’gestation. Whether to give IUT to an affected fetus depends not juston fetal Hb level, but also on ultrasound signs of severe anemia,oxygen and pH status.-
dc.languageeng-
dc.publisherJohn Wiley & Sons Ltd. The Journal's web site is located at http://www.interscience.wiley.com/jpages/0960-7692/-
dc.relation.ispartofUltrasound in Obstetrics and Gynecology-
dc.rightsUltrasound in Obstetrics and Gynecology. Copyright © John Wiley & Sons Ltd.-
dc.rightsSpecial Statement for Preprint only Before publication: 'This is a preprint of an article accepted for publication in [The Journal of Pathology] Copyright © ([year]) ([Pathological Society of Great Britain and Ireland])'. After publication: the preprint notice should be amended to follows: 'This is a preprint of an article published in [include the complete citation information for the final version of the Contribution as published in the print edition of the Journal]' For Cochrane Library/ Cochrane Database of Systematic Reviews, add statement & acknowledgement : ‘This review is published as a Cochrane Review in the Cochrane Database of Systematic Reviews 20XX, Issue X. Cochrane Reviews are regularly updated as new evidence emerges and in response to comments and criticisms, and the Cochrane Database of Systematic Reviews should be consulted for the most recent version of the Review.’ Please include reference to the Review and hyperlink to the original version using the following format e.g. Authors. Title of Review. Cochrane Database of Systematic Reviews 20XX, Issue #. Art. No.: CD00XXXX. DOI: 10.1002/14651858.CD00XXXX (insert persistent link to the article by using the URL: http://dx.doi.org/10.1002/14651858.CD00XXXX) (This statement should refer to the most recent issue of the Cochrane Database of Systematic Reviews in which the Review published.)-
dc.titleDoes in-utero transfusion for homozygous α0-thalassemia depend on hemoglobin level alone?-
dc.typeConference_Paper-
dc.identifier.emailLi, TKT: liktt@HKUCC-COM.hku.hk-
dc.identifier.emailLeung, KY: leungky1@ha.org.hk-
dc.identifier.emailTang, MHY: tanghym@ha.org.hk-
dc.identifier.emailChan, VNY: vnychana@hkucc.hku.hk-
dc.identifier.authorityTang, MHY=rp01701-
dc.identifier.authorityChan, VNY=rp00320-
dc.identifier.doi10.1002/uog.7261-
dc.identifier.hkuros181264-
dc.identifier.volume34-
dc.identifier.issueS1-
dc.identifier.spage249 Abstract no. P18.08-
dc.identifier.epage249 Abstract no. P18.08-
dc.publisher.placeUnited Kingdom-

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